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Updated: Aug 1, 2025
Assessing the Development of Murine Plasmacytoid Dendritic Cells in Peyer's Patches Using Adoptive Transfer of Hematopoietic Progenitors
Published on: March 17, 2014
Plasmacytoid dendritic cell neoplasms
Yoo Jin Lee1, Youjin Kim1, Sang Hyuk Park2
1Department of Hematology and Oncology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.
Insights
Plasmacytoid dendritic cell (pDC) neoplasms include mature pDC proliferation and blastic pDC neoplasm (BPDCN). BPDCN is an aggressive malignancy with emerging targeted therapies like tagraxofusp.
Area of Science:
- Immunology
- Hematology
- Oncology
Background:
- Plasmacytoid dendritic cells (pDCs) are key immune modulators, producing type I interferons.
- pDC neoplasms are broadly classified into mature pDC proliferation (MPDCP) and blastic pDC neoplasm (BPDCN).
- MPDCP is linked to chronic myelomonocytic leukemia, while BPDCN is an aggressive malignancy affecting multiple organs.
Purpose of the Study:
- To provide a comprehensive review of pDC neoplasms.
- To detail the characteristics and immunophenotype of BPDCN.
- To discuss current and emerging therapeutic strategies for BPDCN.
Main Methods:
- Literature review of pDC neoplasms.
- Analysis of clinical and pathological features of BPDCN.
- Summary of treatment modalities, including historical and novel targeted agents.
Main Results:
- BPDCN presents with diverse skin lesions and involves skin, bone marrow, lymphatic organs, and CNS.
- Typical BPDCN immunophenotype includes CD4, CD56, CD123, and specific pDC markers.
- Treatment historically involved acute leukemia regimens and transplantation; targeted therapies are emerging.
Conclusions:
- BPDCN is a distinct and aggressive myeloid malignancy.
- Understanding the immunophenotype is crucial for BPDCN diagnosis.
- Targeted therapies, particularly those against CD123, show promise for BPDCN treatment.
Abstract:
Plasmacytoid dendritic cells (pDCs) are type I interferon-producing cells that modulate immune responses. There are two types of pDC neoplasms: 1) mature pDC proliferation (MPDCP) associated with myeloid neoplasm and 2) blastic pDC neoplasm (BPDCN). MPDCP is a clonal expansion of mature pDCs that is predominantly associated with chronic myelomonocytic leukemia. In contrast, BPDCN is a clinically aggressive myeloid malignancy involving the skin, bone marrow, lymphatic organs, and central nervous system. There are various types of skin lesions, ranging from solitary brown or violaceous to disseminated cutaneous lesions, which often spread throughout the body. The expression of CD4, CD56, CD123, and pDC markers (TCL-1, TCF4, CD303, and CD304, etc.) are typical immunophenotype of BPDCN. Historically, BPDCN treatment has been based on acute leukemia regimens and allogeneic hematopoietic cell transplantation in selected patients. Recent advances in molecular biology and genetics have led to the development of targeted agents, such as tagraxofusp (a recombinant fusion protein targeting CD123), anti-CD123 CAR-T cells, XmAb14045, and IMGN632. Lastly, this review provides a comprehensive overview of pDC neoplasms.
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